Impaired postural stability as a marker of premanifest Huntington's disease.
Impaired postural stability as a marker of premanifest Huntington's disease.
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姿势稳定性受损是亨廷顿病前期的标志。
DOI：
10.1002/mds.23309
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发表时间：
2010
期刊：
影响因子：
--
通讯作者：
Gilbert,PaulE
中科院分区：
文献类型：
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作者：
Salomonczyk,Danielle;Panzera,Robert;Pirogovosky,Eva;Goldstein,Jody;Corey-Bloom,Jody;Simmons,Roger;Gilbert,PaulE
在携带亨廷顿舞蹈病（HD）突变但尚未表现出症状的个体中，观察到精细运动控制的细微变化，称为先兆HD （preHD）。然而，很少有研究检查了这一人群的大运动障碍。本研究旨在利用计算机动态姿势照相技术，探讨在HD的前期和显性阶段，感觉参与在维持姿势稳定性中的作用。11名HD参与者，22名preHD被分为“preHD近”（距离估计的临床发病时间<5年）和“preHD远”（距离估计的临床发病时间<5年），以及17名非基因携带者（NGC）完成了感觉组织测试（SOT），以评估前庭、视觉和体感信息系统退化时的姿势控制。在包括基线在内的所有条件下，HD组比NGC组和preHD Far组表现出更大的姿势摇摆，在操纵感觉信息时，HD组比preHD Near组表现出更大的姿势摇摆。当视觉和体感信息退化，只有前庭信息可用且可靠地维持姿势稳定时，preHD近组比preHD远组表现出更大的姿势摇摆。这项研究的结果强调，在预估疾病发病前5年，hd患者在面对变化的感觉条件时存在细微的姿势缺陷。研究结果表明，SOT可能是HD前期早期运动障碍和随后表型转化为HD的一个高度敏感的指标。©2010运动障碍协会
Subtle changes in fine motor control have been observed in individuals who carry the Huntington's disease (HD) mutation but have not yet manifested symptoms, referred to as premanifest HD (preHD). However, few studies have examined gross motor impairments in this population. This study sought to examine the role of sensory involvement in maintaining postural stability during the premanifest and manifest stages of HD using computerized dynamic posturography. Eleven HD participants, 22 preHD subdivided into “preHD Near” (<5 years from estimated clinical onset) and “preHD Far” (>5 years from estimated clinical onset), and 17 nongene carriers (NGC) completed a sensory organization test (SOT) to assess postural control when vestibular, visual, and somatosensory information was systematically degraded. The HD group demonstrated greater postural sway than the NGC and preHD Far groups on all conditions including baseline, and greater postural sway than the preHD Near group when sensory information was manipulated. The preHD Near group showed significantly greater postural sway than the preHD Far group when visual and somatosensory information was degraded and only vestibular information was available and reliable for maintaining postural stability. The results of this study highlight subtle postural deficits in the face of changing sensory conditions in preHD up to 5 years before estimated disease onset. The findings suggest that the SOT may be a highly sensitive indicator of early motor impairment and subsequent phenoconversion to manifest HD in preHD. © 2010 Movement Disorder Society